angioimmunoblastic t cell lymphoma presenting as giant kidneys a case report

Case reportAngioimmunoblastic T-cell lymphoma presenting as giant kidneys: a case report Ori Argov1*, Gideon Charach1, Moshe Weintraub1 and Alexander Shtabsky2 Addresses: 1 Department of

Case report

Angioimmunoblastic T-cell lymphoma presenting as giant kidneys:

a case report

Ori Argov1*, Gideon Charach1, Moshe Weintraub1 and Alexander Shtabsky2

Addresses: 1 Department of Internal Medicine “C”, Tel-Aviv Sourasky Medical Center, 6 Weizman St, Tel Aviv 64239, Israel, and The Sackler Faculty

of Medicine, Tel-Aviv University, Tel-Aviv, Israel

2 Pathology Institute, Tel-Aviv Sourasky Medical Center, 6 Weizman St, Tel Aviv 64239, Israel, and The Sackler Faculty of Medicine,

Tel-Aviv University, Tel-Aviv, Israel

Email: OA* - oriargov@netvision.net.il; GC - gideonc@tasmc.health.gov.il; MW - wmoshe@post.tau.ac.il; AS - shtabsky@gmail.com

* Corresponding author

Received: 29 January 2009 Accepted: 14 April 2009 Published: 14 September 2009

Journal of Medical Case Reports 2009, 3:9258 doi: 10.4076/1752-1947-3-9258

This article is available from: http://casesjournal.com/casesjournal/article/view/9258

© 2009 Argov et al.; licensee Cases Network Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction: Angioimmunoblastic T-cell lymphoma is a rare form of tumor of the lymph nodes or

lymphoid tissue In this report we describe an unusual presentation of angioimmunoblastic T-cell

lymphoma consisting of giant kidneys with no nephrotic syndrome

Case presentation: A 46-year-old Arabic man from Gaza was hospitalized in our ward due to

abdominal pain and a weight loss of 20 kg during the preceding two months The results of the

physical examination and laboratory tests raised the possibility of neoplastic disease A computerized

tomographic scan of the abdomen showed huge kidneys, and a kidney biopsy showed infiltration by

lymphocytes and eosinophils The genetic examination revealed T-cell lymphoma Diagnosis was

made by a lymph node biopsy, which shows typical findings of angioimmunoblastic T-cell lymphoma

Conclusions: Angioimmunoblastic T-cell lymphoma can present with huge kidneys without

nephrotic syndrome

Introduction

Angioimmunoblastic T-cell lymphoma is rare, occurring in

only 1% of all cases of lymphoma It is characterized by

the loss of lymphoid architecture, with a pleomorphic

cellular infiltrate and proliferation of small blood vessels

Patients usually present with B symptoms (weight loss,

sweating and fever), generalized lymphadenopathy, skin

rash, polyclonal hypergammaglobulinemia, autoimmune

disorders (for example, Coombs-positive hemolytic

ane-mia), or infections Diagnosis can be made by a lymph

node biopsy which will show infiltration of small lymphocytes, plasma cells, immunoblasts, histiocytes, and often eosinophils The malignant cells are CD4+ abT cells with TCR b and g rearrangements that may express CD10 and Bcl-6 Treatment is through doxorubicin-based regimens We report the case of a man who sought medical advice due to weight loss and abdominal pain, and whose unusual presentation of angioimmunoblastic T-cell lym-phoma consisted of giant kidneys with no nephrotic syndrome

Case presentation

A 46-year-old Arabic man from Gaza was hospitalized in

our ward due to abdominal pain and a weight loss of 20 kg

during the preceding two months His medical history did

not contribute any useful information for the diagnosis and

he denied fever or night sweats His physical examination

yielded normal results except for diffuse abdominal

tenderness His body temperature was 37.5°C during the

first few days of hospitalization Laboratory tests showed

marked eosinophilia (30%), hyperglobulinemia (72 mg/l),

and mild renal dysfunction (creatinine 1.7 mg/dl) There

was no anemia (hemoglobin 13 mg/dl), leukocytosis,

disturbance of liver function or elevation of C-reactive

protein levels Blood and urine cultures tested negative as did

serologic tests for hepatitis,Rickettsiae and other zoonotic

infections PPD (tuberculin) tests were negative (twice) as

was an HIV test, and we ruled out parasitic infection The

results of a complete panel of laboratory examinations for

autoimmune diseases came back negative, and connective

tissue disease was ruled out as well A 24-hour urine

collection ruled out nephrotic syndrome, and a microscopic

examination of the urine was normal The

hyperglobuline-mia was found to be polyclonal so the possibility of multiple

myeloma was excluded

At this point, the combination of weight loss, eosinophilia

and hyperglobulinemia raised the possibility of neoplastic

disease A computerized tomographic (CT) scan of the

abdomen (Figure 1) showed huge kidneys measuring

22 cm in length Numerous lymph nodes were found to be enlarged in the mediastinum, inguinal area and along the aorta A positron emission tomographic CT (PET-CT) scan showed a diffuse nodular lymphoproliferative disease above and below the diaphragm, involving giant kidneys (Figure 2) A kidney biopsy showed effacement of the renal structure by diffuse leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes (Figure 3) A polymerase chain reaction analysis of the gamma T-cell receptor rearrangement showed mono-clonality of the T cells, which raised the possibility of T-cell lymphoma Infiltration by lymphocytes stained mostly for CD3 (T lymphocytes) A biopsy of an inguinal lymph node was remarkable for obliteration of the node architecture (Figure 4) The paracortical area was infiltrated by cells that were positive for CD3 and CD4 (Figure 5) Bone marrow biopsy showed eosinophilia without lymphatic aggregates The constellation of eosi-nophilia, hyperglobulinemia, generalized lymphadeno-pathy, giant kidneys and the findings in the lymph node biopsy were consistent with the diagnosis of angioimmu-noblastic T-cell lymphoma

The patient underwent a five-session course of cyclopho-sphamide, doxorubicin, vincristine and prednisone After this, the patient experienced considerable improvement in the function of his kidneys Creatinine levels dropped to 1.2 mg/dl, and a repeated CT scan showed that the

Figure 1 Abdominal computerized tomographic scan

showing kidneys measuring 22 cm in length

Figure 2 A positron emission tomographic scan showed a diffuse nodular lymphoproliferative disease involving giant kidneys

kidneys had reduced in length to 13 cm (Figure 6) After 6

months, we re-examined the patient He already appeared

to be in good health and had no health-related

com-plaints Repeated laboratory tests showed decreased

globulin levels, and his blood count showed no elevation

in the eosinophil level

Discussion

Angioimmunoblastic T-cell lymphoma represents 1% of

all lymphomas It was first described in 1974 [1] and

called angioimmunoblastic lymphadenopathy with

dysproteinemia Later, when genetic unity was found in

the T-cell receptors, it was categorized as one of the

peripheral (mature) T-cell lymphoma (PTCL) group This disease is usually diagnosed among men who are 40 years

or older It is characterized by B symptoms (fever of over 38°C, drenching night sweats or unintentional weight loss), lymphadenopathy, polyclonal hyperglobulinemia and eosinophilia [2] Nephrotic syndrome in angioimmu-noblastic lymphoma is uncommon and mentioned only

in isolated case reports [3,4] The median survival is about

30 months and the cause of death is usually due to

Figure 3 Renal biopsy shows parenchyma effacement by the

diffuse leukocytic infiltrate

Figure 4 Biopsy of a lymph node shows obliteration of the

node architecture

Figure 5 Lymph node biopsy shows CD3 positive infiltrate

Figure 6 Abdominal computerized tomographic image after therapy showing normal-sized kidneys

infections During biopsy, an involved lymph node will

show destruction of the architecture and an infiltration

composed of lymphocytes and eosinophils

Immunophe-notyping will show mature T cells that are T-helper cells

Therapy is composed of doxorubicin-based regimens

Complete response rates are 64% [5]

Conclusion

The unusual presentation of the disease consisting of giant

kidneys with no nephrotic syndrome in our patient is

uncommon He also did not have the splenomegaly and

skin manifestations found in half of affected patients [2]

This case shows that angioimmunoblastic lymphoma can

present with huge kidneys without nephrotic syndrome

This uncommon presentation of this rare disease together

with the remarkable findings on the CT scan and PET-CT

make this an educational case

Abbreviations

CT, computerized tomography; PPD, purified protein

derivative; PTCL, peripheral (mature) T-cell lymphoma

Consent

Written informed consent was obtained from the patient

for publication of this case report and any accompanying

images A copy of the written consent is available for

review by the Editor-in-Chief of this journal

Competing interests

The authors declare that they have no competing interests

Authors ’ contributions

OA attended the patient, collected data and wrote

the manuscript GC attended the patient and revised the

manuscript MW (head of department) attended the

patient, and conceptualized the peculiarity of the case

AS revised the pathological specimens

Acknowledgement

Esther Eshkol is thanked for editorial assistance

References

1 Frizzera G, Moran EM, Rappaport H: Angio-immunoblastic

lymphadenopathy with dysproteinaemia Lancet 1974,

1:1070-1073.

2 Siegert W, Nerl C, Agthe A, Engelhard M, Brittinger G, Tiemann M,

Lennert K, Huhn D: Angioimmunoblastic lymphadenopathy

(AILD)-type T-cell lymphoma: prognostic impact of clinical

observations and laboratory findings at presentation The

Kiel Lymphoma Study Group Ann Oncol 1995, 6:659-664.

3 De Samblanx H, Verhoef G, Zachée P, Vandenberghe P: A male with

angioimmunoblastic T-cell lymphoma and proliferative

glo-merulonephritis Ann Hematol 2004, 83:455-459.

4 Goto A, Takada A, Yamamoto S, Notoya A, Mukai M:

Angioimmu-noblastic T-cell lymphoma with renal involvement: a case

report of direct bilateral kidney invasion by lymphoma cells.

Ann Hematol 2004, 83:731-732.

5 Siegert W, Agthe A, Griesser H, Schwerdtfeger R, Brittinger G, Engelhard M, Kuse R, Tiemann M, Lennert K, Huhn D: Treatment of angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma using prednisone with or without the COP-BLAM/IMVP-16 regimen A multicenter study Kiel Lym-phoma Study Group Ann Intern Med 1992, 117:364-370.

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